Pediatric Thymoma Follow-up

  • Author: Cecilia P Mikita, MD, MPH; Chief Editor: Harumi Jyonouchi, MD   more...
 
Updated: Aug 12, 2010
 

Complications

  • Structural problems, such as compression syndromes that involve the bronchi or lungs or superior vena cava syndrome (SVCS), can occur from local spread of benign thymoma, from thymic cysts, or from thymic carcinoma. Presenting symptoms may include chest pain, SVCS, dyspnea, dysphagia, and cough.
  • Areas of benign thymoma can become highly vascular or necrotic and lead to bleeding.
  • Clinical manifestations include paraneoplastic syndromes and immunodeficiency.
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Prognosis

  • See Pathophysiology.
  • Adverse predictive factors include the following:[38, 39]
    • Invasive or metastatic tumor
    • Tracheal or vascular compression
    • Age younger than 30 years
    • Epithelial or mixed histology
    • Tumor size of more than 8 cm[40]
  • The presence of myasthenia gravis (MG) with thymomas is no longer considered a poor prognostic factor and is actually thought to be a favorable prognostic factor.[41]
  • Based on WHO classification, the 5-year and 10-year survival rates are as follows:[42, 43]
    • Type A - 100% and 95%, respectively
    • Type AB - 93% and 90%, respectively
    • Type B1 - 89% and 85%, respectively
    • Type B2 - 82% and 71%, respectively
    • Type B3 - 71% and 40%, respectively
    • Type C - 23% (5-year survival rate)
  • Recurrence of thymoma can occur after resection. A study surgical outcomes after recurrence of thymic epithelial tumors in 67 patients showed an overall survival rate at 10 years of 70% in those undergoing re-resection.[29]
  • For patients with Masaoka stage IVA thymomas, a study of 18 patients reported survival rates at 3 years (91%), 5 years (78%), and 10 years (65%).[44] These patients underwent multimodality therapy, including surgical resection, preoperative chemotherapy, and even postoperative radiation therapy (in select patients).
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Contributor Information and Disclosures
Author

Cecilia P Mikita, MD, MPH  Associate Program Director, Allergy-Immunology Fellowship, Associate Professor of Pediatrics and Medicine, Uniformed Services University of the Health Sciences; Hospital Intern Director, Staff Allergist/Immunologist, Walter Reed Army Medical Center

Cecilia P Mikita, MD, MPH is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology, and Clinical Immunology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Richard A Bickel, MD  Fellow in Allergy/Immunology, Walter Reed Army Medical Center

Richard A Bickel, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

Terry W Chin, MD, PhD  Associate Director, Pediatric Allergy/Immunology/Pulmonology, Miller Children's Hospital, Long Beach Memorial Medical Center; Associate Professor, Department of Pediatrics, University of California, Irvine, School of Medicine

Terry W Chin, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American College of Chest Physicians, American Thoracic Society, California Thoracic Society, Clinical Immunology Society, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David J Valacer, MD  Consulting Staff, Hoffman La Roche Pharmaceuticals

David J Valacer, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American Thoracic Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville School of Medicine

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD  Associate Professor, Division of Pulmonary, Allergy/Immunology, and Infectious Diseases, Department of Pediatrics, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Marion Johnson, MD, to the development and writing of this article.

References

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Table 1. Comparison of the Different Classifications of Thymic Epithelial Tumors[26]
Clinicopathologic ClassificationWHO TypeTerminology of the Histogenetic Classification for the Histologic Subtypes of Thymic Epithelial Tumors
Benign thymomaA



AB



Medullary thymoma



Mixed thymoma



Malignant thymomas,



Category I



B1



B2



B3



Predominantly cortical thymoma



Cortical thymoma



Well-differentiated thymic carcinoma



Malignant thymomas,



Category II



CEpidermoid keratinizing (squamous cell) carcinoma



Epidermoid nonkeratinizing carcinoma



Lymphoepithelioma-like carcinoma



Sarcomatoid carcinoma (carcinosarcoma)



Clear cell carcinoma



Mucoepidermoid carcinoma



Undifferentiated carcinoma



Table. Macchiarini et al (1991)[34]
Cisplatin75 mg/m2 on day 13 courses repeated q3wk
Epirubicin100 mg/m2 on day 1
Etoposide120 mg/m2 on days 1, 3, and 5
Surgery and radiation



in patients with



complete or partial



response to chemotherapy



4500 cGy if complete



resection



6000 cGy if incomplete



resection



Table. Loehrer et al (1997)[35]
Cisplatin50 mg/m22-4 cycles q3wk
Doxorubicin50 mg/m2
Cyclophosphamide500 mg/m2
Followed by radiation54 Gy to the primary tumor and lymph nodes
Table. Venuta et al (1997)[36]
Cisplatin75-100 mg/m2 on day 1Repeated q3wk 3 times before surgery and 2 or 3 times after surgery
Epirubicin hydrochloride100 mg/m2 on day 1
Etoposide120 mg/m2 on days 1, 3, and 5
Postoperative radiation in patients with radical resection30 GyDelivered in 3 wk with 5 fractions per wk
Postoperative radiation in



patients with incomplete resection



50 GyDelivered in 5 wk with 5 fractions per wk
Table. Palmieri et al (1999)[37]
Octreotide1.5 mg/d SCIn patients shown to have



somatostatin receptors



Lanreotide30 mg/d SC q14dSwitch to this longer-acting



somatostatin analogue or depot form of octreotide if short-acting octreotide



is well tolerated



Prednisone0.6 mg/kg/d PO



for 3 mo, then



decreasing to 0.2 mg/kg



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